Disease relevance of Exophthalmos

• Cyclosporin given to two patients with Graves' ophthalmopathy produced a rapid objective improvement in both, including restoration of ocular-muscle function and visual acuity, improvement in exophthalmos, and reduction in orbital muscle swelling judged by computed tomographic scan [1].
• Recurrent Churg-Strauss vasculitis. With exophthalmos, hearing loss, nasal obstruction, amyloid deposits, hyperimmunoglobulinemia E, and circulating immune complexes [2].
• MAIN OUTCOME MEASURES: Need for surgery, steroid therapy, volume of extraocular muscles and fat, proptosis, area of diplopia fields and range of extraocular muscle motion, volume changes after decompression and correlations of eye findings with serum TSI levels, retinal status [3].
• Bone fragility, craniosynostosis, ocular proptosis, hydrocephalus, and distinctive facial features: a newly recognized type of osteogenesis imperfecta [4].
• Indium-111 octreotide in Graves' disease and in the evaluation of active exophthalmos [5].

Psychiatry related information on Exophthalmos

• The cessation of methadone or morphine but not saline treatment followed by naloxone resulted in graded signs including wet dog shakes, escape attempts, self-stimulation and body weight loss and quantal signs including diarrhea, ear blanching, exophthalmos, ptosis, tachypnea and teeth chattering [6].

High impact information on Exophthalmos

• Crouzon syndrome, an autosomal dominant condition characterized by craniosynostosis, ocular proptosis and midface hypoplasia, is associated with mutations in fibroblast growth factor receptor 2 (FGFR2) (refs 1-3) [7].
• Though progression of exophthalmos was noted in all groups, the group that received propylthiouracil demonstrated the greatest progression of exophthalmos [8].
• Somatomedin C in pathogenesis of malignant exophthalmos of endocrine origin [9].
• In addition, Abca5(-)(/)(-) mice also exhibited exophthalmos and collapse of the thyroid gland [10].
• Letter: Exophthalmos associated with systemic lupus erythematosus [11].

Chemical compound and disease context of Exophthalmos

• The exophthalmogenic factor and its parent thyrotropin molecule can stimulate adenylate cyclase activity in retro-orbital tissue membranes from guinea pigs, a mammalian model of exophthalmos; its polypeptide components have little or no such activity [12].
• METHODS: Sixteen patients with Graves orbitopathy were studied before and 3 to 6 months after translid (6 patients) and coronal (10 patients) orbital decompression surgery for disfiguring proptosis [13].
• Exophthalmos and elevated thyroxine levels in association with lithium therapy [14].
• Daily replacement therapy with T4 (15mug/kg body wt) failed to alter significantly the exophthalmos which developed, even when replacement was initiated prior to the alterations of thyroid gland function; this observation tends to eliminate thyroid hormone deficiency per se as the causal event in exophthalmos [15].
• METHODS: Five patients who developed symptomatic unilateral proptosis after steroid injection were studied and the literature was reviewed [16].

Biological context of Exophthalmos

• Other presenting features were diplopia, proptosis, poor visual acuity, visual field defects, pupillary abnormalities, fundal changes and altered colour vision [17].
• We present a case of Pfeiffer syndrome prenatally diagnosed at 20 weeks' gestation, in which the sonographic features of craniosynostosis, hypertelorism associated with an extreme proptosis, and broad thumb led to the diagnosis, confirmed after termination of pregnancy by dysmorphological, pathological and radiological evaluation [18].
• However when proptosis is severe, with raised intraocular pressure, severe chemosis and danger of blindness, then the choice of therapy is controversial: rapid decompression by surgical means or use of high doses of prednisolone [19].
• For treatment of patients with complex craniosynostosis, increased intracranial pressure, severe exophthalmos, and apnea attacks are important indications for operative intervention [20].

Anatomical context of Exophthalmos

• Ocular examination features studied included assessment of visual acuity, optic nerve appearance and function, proptosis, strabismus, eyelid position, and exposure keratopathy [21].
• Beare-Stevenson syndrome is a craniofacial syndrome consisting of a specific pattern of craniosynostosis resulting in a cloverleaf skull deformity and hydrocephalus, down-slanting palpebral fissures, proptosis, hypertelorism, strabismus, dysmorphic ears, choanal atresia, cleft palate, cutis gyratum, acanthosis nigricans, and abnormal genitalia [22].

Gene context of Exophthalmos

• Goiter, menstrual disorders, short stature, constipation, nervousness and exophthalmos have been reported as the most recurrent clinical features of HT [23].
• Axial myopia. A neglected cause of proptosis [24].
• BACKGROUND: Progressive exophthalmos from dysthyroid orbitopathy may result in marked disfigurement [25].
• The triad of exophthalmos, pretibial myxedema, and acropachy occurs in less than 1% of patients [26].
• A review of 10 cases of metastatic hepatocellular carcinoma to the eye and orbit disclosed painful proptosis as the most common clinical sign of hepatocellular carcinoma metastatic to the orbit [27].

Analytical, diagnostic and therapeutic context of Exophthalmos

• Ten patients who had unilateral proptosis ranging from 2.5 to 4.0 mm were found to have axial myopia confirmed by ultrasonography and, in one case, by computerized tomography [24].
• Seven patients with malignant exophthalmos were treated with steroid therapy, supervoltage orbital radiotherapy or plasmapheresis [28].
• CONCLUSION: Relative to the coronal approach, swinging eyelid decompression results in at least the same proptosis reduction, no greater motility disturbance, and a shorter hospitalization time [29].
• However, cervical sympathectomy or guanethidine pretreatment only partially blocked the increase in PD or exophthalmos [30].

References